Description:
<jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>Antimicrobial peptides have attracted much attention as a member of disease‐associated molecules in systemic sclerosis (<jats:styled-content style="fixed-case">SS</jats:styled-content>c), which is pathologically characterized by immune abnormalities, vasculopathy and tissue fibrosis.</jats:p></jats:sec><jats:sec><jats:title>Objective</jats:title><jats:p>To investigate the potential contribution of one of the antimicrobial peptide psoriasin to the development of <jats:styled-content style="fixed-case">SS</jats:styled-content>c.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>Psoriasin expression in the skin samples and sera derived from <jats:styled-content style="fixed-case">SS</jats:styled-content>c patients and its correlation with clinical parameters were analysed. Psoriasin expression was evaluated by immunohistochemistry with skin samples from <jats:styled-content style="fixed-case">SS</jats:styled-content>c patients and healthy controls. Serum levels of psoriasin were determined by enzyme‐linked immunosorbent assay in 51 <jats:styled-content style="fixed-case">SS</jats:styled-content>c patients and 19 healthy controls and assessed for the association with clinical symptoms.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>The expression of psoriasin was elevated in the epidermis of <jats:styled-content style="fixed-case">SS</jats:styled-content>c lesional skin. Serum psoriasin levels were higher in <jats:styled-content style="fixed-case">SS</jats:styled-content>c patients, especially in diffuse cutaneous <jats:styled-content style="fixed-case">SS</jats:styled-content>c patients with disease duration of <6 years, than in healthy controls. With respect to clinical association, <jats:styled-content style="fixed-case">SS</jats:styled-content>c patients with interstitial lung disease, telangiectasia and pitting scars had significantly augmented levels of serum psoriasin than those without each of these symptoms. In the subgroup of patients with interstitial lung disease, the elevation of serum psoriasin levels was associated with higher ground‐glass opacity scores. Furthermore, serum psoriasin levels were decreased after the treatment with intravenous cyclophosphamide pulse as compared to baseline values.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>Our findings indicate a possible contribution of psoriasin to the development of clinical symptoms associated with vascular and epithelial abnormalities and inflammation in <jats:styled-content style="fixed-case">SS</jats:styled-content>c, further supporting the roles of antimicrobial peptides in the <jats:styled-content style="fixed-case">SS</jats:styled-content>c pathogenesis.</jats:p></jats:sec>